A 43-year-old woman has extensive nevus of Ota associated with ipsilateral sensorineural deafness. She has unilateral pigmentary involvement of the upper portion of the face, palate, nasal ala, posterior portion of the concha auriculae, external ear canal, and tympanic membrane. She has increased pigmentation of the sclera, conjunctiva, iris, and retina of the left eye. Melanoma may develop in the skin, eye, and brain in these patients and probably occurs more frequently in white than in nonwhite patients. Melanocytes are a normal component of the petrous bones of the ear; and the melanocytes are increased in this patient, causing her otological problems.